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11/17. Signal changes on MRI and increases in reactive microgliosis, astrogliosis, and iron in the putamen of two patients with multiple system atrophy.

    A correlation of clinical, MRI, and neuropathological data is reported in two patients with multiple system atrophy (MSA). On MRI, patient 1 showed striatal atrophy, reduction of T2 relaxation times within most of the putamen, and a band of hyperintense signal changes in the lateral putamen. In patient 2, MRI disclosed only shortening of the T2 signal in the putamen. immunohistochemistry showed pronounced reactive microgliosis and astrogliosis in the affected brain regions. In patient 1, the area with the most pronounced microgliosis and astrogliosis most likely correlated with the area of hyperintense signal changes on MRI. This area also contained the highest amount of ferric iron, which was increased in the putamen of patient 1 but not patient 2. It is unlikely that the hypointense signal changes in the putamen are due to an increase of iron alone. Reactive microglial and astroglial cells may play a part in the pathogenesis of MSA.
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12/17. PET analysis of a case of cerebrotendinous xanthomatosis presenting hemiparkinsonism.

    We describe a 34-year-old Japanese woman presenting gait difficulty and Achilles tendon swelling. The patient was diagnosed as having cerebrotendinous xanthomatosis (CTX) based on the high serum cholestanol level and diminished enzymatic activity of 27-hydroxylase of fibroblasts from her skin. Her clinical presentation was atypical regarding the presence of hemiparkinsonism and absence of apparent cataract, dementia, and cerebellar ataxia. Although MRI studies could not detect any abnormality in the basal ganglia or midbrain, PET analysis using [18F]-6-fluoro-L-dopa revealed reduced uptake of dopamine into the putamen, suggesting the impairment of presynaptic dopaminergic neurons.
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13/17. Hemiparkinsonism following haemorrhage in the contralateral substantia nigra.

    We report a patient with right hemiparkinsonism following haemorrhage in the left substantia nigra. The hemiparkinsonism responded to treatment with trihexyphenidyl hydrochloride and deteriorated after temporary discontinuation of the drug. Single photon emission computed tomography using technetium 99m d, l-hexamethylpropyleneamine oxide showed reduced uptake in the left putamen, globus pallidus and thalamus.
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14/17. Postencephalitic stereotyped involuntary movements responsive to L-Dopa.

    In 1954, at the age of 5 years, our patient had an encephalitic syndrome associated with a prolonged lethargic state. After this episode, he developed a severe parkinsonian syndrome that, after a few years, was associated with axial dystonia and stereotyped abnormal movements of the upper limbs. This complex and progressive extrapyramidal syndrome had many similarities to the encephalitis lethargica as described by von Economo. Results of cerebral computed tomography and magnetic resonance imaging were normal. Fluorodopa positron emission tomography showed a significant bilateral reduction of tracer accumulation in both putamen, similar to that observed in patients with idiopathic Parkinson's disease. However, in this patient, treatment with L-Dopa suppressed all akinetic, dystonic and dyskinetic symptoms. The effectiveness of L-Dopa was abolished by administration of a D2 antagonist and was fully reproduced by a D2 agonist. In conclusion, this patient presented a complex postencephalitic, extrapyramidal syndrome, with akinetic symptoms and involuntary movements. These symptoms appeared to be related to a limited lesion of the dopaminergic neurons of the zona compacta of the substantia nigra.
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15/17. Parkinsonism associated with acute intracranial hematomas: an [18F]dopa positron-emission tomography study.

    We present the case of a 36-year-old woman with a right temporal hematoma and an overlying subdural hematoma following rupture of a right middle cerebral artery aneurysm. Three weeks after recovering consciousness, she developed a levodopa-responsive parkinsonian syndrome involving the right limbs. A year after the vascular event, she reported subjective improvement in her parkinsonism, which has remained stable since. 18F-dopa positron-emission tomography showed a marked reduction of uptake in the left putamen, raising the possibility that the intracranial hemorrhage unmasked latent Parkinson's disease. To the best of our knowledge, this is the first case of parkinsonism associated with spontaneous acute intracerebral and subdural hematomas.
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16/17. X-linked dystonia-deafness syndrome.

    We report a family with early-onset deafness and progressive dystonia exclusively involving males over two successive generations. There is also evidence of cognitive impairment and corticospinal tract involvement. The pedigree suggests an X-linked inheritance. A similar family was originally described by Scribanu and Kennedy. Tranebjaerg et al. have recently reported two other families with linkage to Xq22 and also proposed a novel X-linked candidate gene. These findings support the existence of a distinct neurodegenerative syndrome principally characterized by early-onset deafness and progressive dystonia. Neuropathology of one case showed a mosaic pattern of neuronal loss and gliosis in the caudate and putamen suggesting that this pattern is not restricted to XDP or Lubag.
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17/17. magnetic resonance imaging and 11C-N-methylspiperone/positron emission tomography studies in a patient with the interval form of carbon monoxide poisoning.

    Magnetic resonance (MR) and (11)C-N-methylspiperone ((11)C-NMSP)/positron emission tomography (PET) imagings were repeatedly performed in a 50-year-old man with the interval form of carbon monoxide (CO) poisoning. In MR images obtained when delayed neuropsychiatric symptoms developed (two months after poisoning), the inner segments of the bilateral globus pallidus appeared as high signal intensities in the T1-weighted and low signal intensities in the T2-weighted images, suggesting prior focal hemorrhage in these areas. A PET study with (11)C-NMSP performed at that time showed an increase in dopamine D2 receptor binding in the caudate and putamen. Treatment with bromocriptine was very effective and five months after the poisoning, MR and (11)C-NMSP/PET images showed improvement, concomitantly with the disappearance of the neuropsychiatric symptoms.
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